Incidence and Mortality of Physician-Diagnosed Primary Sjögren Syndrome: Time Trends Over a 40-Year Period in a Population-Based US Cohort.

OBJECTIVE: To estimate the incidence and mortality rates, and their evolution over time, of physician-diagnosed primary Sjögren syndrome (pSS) in residents of Olmsted County, Minnesota. PATIENTS AND METHODS: Medical records of patients with a diagnosis or suspicion of SS in Olmsted County from January 1, 2006, through December 31, 2015, were reviewed to identify incident cases of pSS (defined by physician diagnosis). These cases were combined with those from a 1976 through 2005 incident cohort (n=111) from the same population. Incidence rates were age and sex adjusted to the 2010 US white population. Survival rates were compared with the expected rates in the population of Minnesota. RESULTS: With 61 incident cases of pSS diagnosed in Olmsted County from 2006 through 2015, the total cohort included 172 patients with incident pSS from 1976 through 2015. Of the 172 patients, 151 (88%) were women and 161 (94%) were white, with a mean ± SD age at diagnosis of 58.3±16.7 years. The average age- and sex-adjusted annual incidence for 2006 through 2015 was 5.9 per 100,000 population (95% CI, 4.4-7.4 per 100,000 population), and the overall incidence for the entire period was 5.8 per 100,000 (95% CI, 4.9-6.6 per 100,000). The incidence increased with calendar time over the 40-year period (P=.005). There was no difference in mortality in the pSS cohort compared with expected (standardized mortality ratio, 1.15; 95% CI, 0.86-1.50). CONCLUSION: The average annual incidence of pSS in this population-based cohort was 5.8 per 100,000, with a progressive increase over the 40 years of the study. Overall survival of patients with pSS was not different from that of the general population.

Prevalence of Primary Sjögren's Syndrome in a US Population-Based Cohort.

OBJECTIVE: To report the point prevalence of primary Sjögren's syndrome (SS) in the first US population-based study. METHODS: Cases of all potential primary SS patients living in Olmsted County, Minnesota, on January 1, 2015, were retrieved using Rochester Epidemiology Project resources, and ascertained by manual medical records review. Primary SS cases were defined according to physician diagnosis. The use of diagnostic tests was assessed and the performance of classification criteria was evaluated. The number of prevalent cases in 2015 was also projected based on 1976-2005 incidence data from the same source population. RESULTS: A total of 106 patients with primary SS were included in the study: 86% were female, with a mean ± SD age of 64.6 ± 15.2 years, and a mean ± SD disease duration of 10.5 ± 8.4 years. A majority were anti-SSA positive (75%) and/or anti-SSB positive (58%), but only 22% met American-European Consensus Group or American College of Rheumatology criteria, because the other tests required for disease classification (ocular dryness objective assessment, salivary gland functional or morphologic tests, or salivary gland biopsy) were rarely performed in clinical practice. According to the physician diagnosis, the age- and sex-adjusted prevalence of primary SS was 10.3 per 10,000 inhabitants, but according to classification criteria, this prevalence was only 2.2 per 10,000. The analysis based on previous incidence data projected a similar 2015 prevalence rate of 11.0 per 10,000. CONCLUSION: The prevalence of primary SS in this geographically well-defined population was estimated to be between 2 and 10 per 10,000 inhabitants. Physicians rarely used tests included in the classification criteria to diagnose the disease in this community setting.

Predictors of relapse and treatment outcomes in biopsy-proven giant cell arteritis: a retrospective cohort study.

OBJECTIVE: To evaluate characteristics of relapse, relapse rates, treatment and outcomes among patients with biopsy-proven GCA in a large, single-institution cohort. METHODS: We conducted a retrospective review of all patients with biopsy-proven GCA from 1998 to 2013. Demographic, clinical, laboratory and treatment data at presentation and during follow-up were collected. Comparisons by relapse rate were performed using chi-square tests. Prednisone discontinuation by initial oral dose ≤40 and >40 mg/day was compared using Cox models. RESULTS: The cohort included 286 patients [74% female, mean age at diagnosis 75.0 years (s.d. 7.6), median follow-up 5.1 years). During follow-up, 73 patients did not relapse, 80 patients had one relapse and 133 had two or more relapses. The first relapse occurred during the first year in 50% of patients, by 2 years in 68% and by 5 years in 79%. More patients with established hypertension (P = 0.007) and diabetes (P = 0.039) at GCA diagnosis were in the high relapse rate group ( ≥ 0.5 relapses/year) and more females were in the low or high relapse groups than in the no relapse group (P = 0.034). Patients receiving an initial oral prednisone dose >40 mg/day were able to reach a dose of <5 mg/day [hazard ratio (HR) 1.46 (95% CI 1.09, 1.96)] and discontinue prednisone [HR 1.56 (95% CI 1.09, 2.23)] sooner than patients receiving ≤40 mg/day without an increase in observed glucocorticoid-associated adverse events. CONCLUSION: Females and patients with hypertension or diabetes at GCA diagnosis have more relapses during follow-up. Patients treated with an initial oral prednisone dose >40 mg/day achieved earlier prednisone discontinuation.

Effect of golimumab on carotid atherosclerotic disease measures and cardiovascular events in inflammatory arthritides.

OBJECTIVE: The objective of this study was to assess the effect of golimumab on carotid ultrasound measures and cardiovascular serious adverse events (SAEs) in patients with inflammatory arthritides. METHODS: An exploratory carotid artery ultrasound substudy was performed in the GO-BEFORE study of methotrexate (MTX)-naive rheumatoid arthritis patients, with ultrasounds performed at weeks 0, 24, and 52 to measure common carotid artery intima-media thickness, distensibility coefficient, interadventitial diameter, and plaque count. Cardiovascular SAEs reported over 2 years of follow-up were assessed in 5 golimumab phase 3 clinical trials of patients with rheumatoid arthritis (GO-BEFORE, GO-FORWARD, and GO-AFTER), psoriatic arthritis (GO-REVEAL), and ankylosing spondylitis (GO-RAISE). In GO-BEFORE and GO-FORWARD, patients received placebo + MTX, golimumab 50 mg + MTX, or golimumab 100 mg +/- MTX at baseline and every 4 weeks; in the other 3 trials, patients received placebo or golimumab 50 or 100 mg. RESULTS: The carotid ultrasound substudy showed inconsistent changes in common carotid artery intima-media thickness in the golimumab + MTX groups over time, and there was large variability in the measurements. Increases in interadventitial diameter were observed in the golimumab 100 mg + placebo group, but not in the golimumab + MTX groups. There were no significant differences in the distensibility coefficient and plaque count between the golimumab and placebo groups. Very few patients overall experienced a cardiovascular SAE, and the incidence of cardiovascular SAEs was not statistically different between the golimumab and placebo groups. CONCLUSIONS: The results of the carotid ultrasound substudy were inconclusive, and no increase or decrease in cardiovascular SAEs was observed following 2 years of treatment with golimumab with or without MTX.

History of primary vasculitis in Latin America.

A literature review utilizing Fepafem, Bireme, LiLacs, Scielo Colombia, Scielo Internacional, former MedLine, Pubmed, and BVS Colombia as well as manual searches in the libraries of major Latin American universities was performed to study vasculitis in Latin America. Since 1945, a total of 752 articles have been published by Latin American authors. However, only a minority are devoted to primary vasculitides, and even fewer have been published in indexed journals. Approximately 126 are in OLD, Medline, Pubmed, Bireme, and Scielo. Most publications are from Mexico, followed by Brazil and Colombia. Systematic studies of the epidemiology of primary idiopathic vasculitis are available for a few countries, i.e. Brazil, Mexico, Colombia, Chile, and Peru. Takayasu arteritis and ANCA-associated vasculitis are the best studied forms of vasculitis in Latin America. Interest and expertise in vasculitis is growing in Latin America, as reflected in the increased number of published articles from this region of the world in the last decade. Racial and environmental factors are possibly responsible for the differential expression of various types of primary vasculitis observed in Latin America. With time, the unique features, epidemiology, and better treatment strategies for idiopathic vasculitides in Latin America will emerge.

Are classification criteria for vasculitis useful in clinical practice? Observations and lessons from Colombia.

INTRODUCTION: Idiopathic systemic vasculitis represents a group of clinical entities having non-specific etiology with the common characteristic of acute or chronic inflammatory compromise of the small and large vessels walls, associated with fibrinoid necrosis. OBJECTIVES: To describe the most common inflammatory vascular diseases in a long historical cohort of patients from San Juan de Dios Hospital located in Bogota, Colombia using two different systems and a clinical histopathological correlation format, and to make a comparison between them. METHODS: We reviewed all previously ascertained cases of vasculitis confirmed by biopsy processed between 1953 and 1990, and systematically collected data on all new cases of vasculitis from 1991 to 1997 at the Hospital San Juan de Dios (Bogota-Colombia). The cases were classified in accordance with the Chapel Hill Consensus criteria, and the system proposed by J.T. Lie. RESULTS: Of 165,556 biopsy tissue specimens obtained during this period from our hospital, 0.18% had vasculitis, perivasculitis or vasculopathy. These included 304 histopathological biopsies from 292 patients. Cutaneous leukocytoclastic vasculitis (64 histological specimens) was the most frequently encountered type of "primary" vasculitis followed by thromboangiitis obliterans (38 specimens), and polyarteritis nodosa (24 specimens). Vasculitis associated with connective tissue diseases (33 specimens) and infection (20 specimens) were the main forms of secondary vasculitis, a category that was omitted from the Chapel Hill consensus report. We found that 65.8% of our histopathological diagnoses could not be classified according to the Chapel Hill classification, and 35.2% could not be classified according to the classification of Lie. Only 8.9% of cases remained unclassified by our system after clinical and histological correlation. CONCLUSION: Current vasculitis classification schemes are designed for classification, rather that diagnosis of disease and do not adequately address some common forms of inflammatory vascular diseases, including those of infectious etiology and unusual etiology seen in clinical practice. Based on our clinical experience, we suggest a classification outline which practitioners can use which emphasizes correlation of the clinical picture to the histopathology findings for diagnosis and therapy, which may promote better clinical practice and standardization for clinical trials.

Necrotizing lymphocytic vasculitis limited to the peripheral nerves: report of six cases and review.

Background. The systemic vasculitides are syndromes characterized by inflammation and injury (necrosis or thrombosis) of blood vessels, resulting in clinical manifestations according to the affected vascular bed, but not classically in stocking-glove neuropathy. Objective. To describe a form of primary vasculitis affecting strictly peripheral nerves manifesting as stocking-glove neuropathy. Methods. Case series of 110 patients seen in three centers in Bogotá who presented with symptoms and signs of polyneuropathy and/or were identified with vasculitis affecting only the peripheral nerves, and who underwent sural nerve biopsy. Results. Six patients had a vasculitis affecting only the peripheral nerves diagnosed on sural nerve biopsy which demonstrated a mixed infiltrate of monocytes/macrophages and lymphocytes especially in the small epineurial blood vessels. Over time, all had worsening of symptoms, with grip weakness and motor deficits in the hand and feet. Serologies and acute phase reactants were normal in all patients. Treatment response to immunosuppression was satisfactory in 5 patients; 1 patient had progressive neurologic damage. Conclusions. There is a distinct form of primary vasculitis of the peripheral nervous system characterized by distal sensory polyneuropathy with stocking-glove distribution with good prognosis, few and minor relapses and good response to treatment even after delayed diagnosis.

Historia de las vasculitis primarias en Latinoamérica / History of primary vasculitis in Latinoamerican

En este artículo se hace una revisión extensa sobre las vasculitis primarias y se recopila la información latinoamericana que aparece referen-ciada en las más importantes bases de datos mundiales en inglés, español y portugués, desde el año 1945 hasta septiembre del año 2007. Igualmente se establecieron varios contactos con reumatólogos o médicos interesados en este tópico. Se realizaron búsquedas secundarias de los estudios que aparecieran citados en los artículos seleccionados y se revisaron manualmente abstracts de congresos.

In this paper an extensive review about primary vasculitis and Latino American information that appears referenced in the most important world wide data bases in English, Spanish and Portuguese from 1945 to september 2007, is compiled. Several contacts with rheumatologist or physician interested in this topic were made. Also secondary searches of the studies that appeared mentioned in selected articles were made and the abstracts of congresses were reviewed manually.

Small vessel vasculitis History, classification, etiology, histopathology, clinic, diagnosis and treatment / Vasculitis de pequeños vasos Historia, clasificación, etiología, histopatología, clínica, diagnóstico y tratamiento

Small-vessel vasculitis is a convenient descriptor for a wide range of diseases characterized by vascular inflammation of the venules, capillaries, and/or arterioles with pleomorphic clinical manifestations. The classical clinical phenotype is leukocytoclastic vasculitis with palpable purpura, but manifestations vary widely depending upon the organs involved. Histopathologic examination in leukocytoclastic vasculitis reveals angiocentric segmental inflammation, fibrinoid necrosis, and a neutrophilic infiltrate around the blood vessel walls with erythrocyte extravasation. The etiology of small-vessel vasculitis is unknown in many cases, but in others, drugs, post viral syndromes, malignancy, primary vasculitis such as microscopic polyarteritis, and connective tissue disorders are associated. The diagnosis of small-vessel vasculitis relies on a thorough history and physical examination, as well as relevant antibody testing including antinuclear antibody and antineutrophil cytoplasmic antibody, hepatitis B and C serologies, assessment of complement, immunoglobulins, blood count, serum creatinine, liver function tests, urinalysis, radiographic imaging, and biopsy. The treatment is based primarily on corticosteroid and immunosuppressive agents.

El término vasculitis de pequeños vasos describe a un grupo de enfermedades caracterizadas por inflamación de vénulas, capilares y/o arteriolas con manifestaciones clínicas pleomórficas. El fenotipo clínico clásico es la vasculitis leucocitoclástica con púrpura palpable, pero con manifestaciones que varían ampliamente dependiendo del órgano comprometido. La histología en la vasculitis leucocitoclástica revela una inflamación segmentaria angiocéntrica, necrosis fibrinoide e infiltrado neutrofílico alrededor de los vasos sanguíneos, con extravasación de eritrocitos. La etiología de las vasculitis de pequeños vasos es desconocida, en muchos casos, pero en otros se ha asociado con drogas, síndromes post virales, neoplasias, vasculitis primarias como la poliarteritis microscópica, y enfermedades del tejido conjuntivo. El diagnóstico de las vasculitis de pequeños vasos se basa en la historia clínica y el examen físico, así como con estudio de anticuerpos como los anticuerpos antinucleares y los anticuerpos contra el citoplasma de los neutrófilos, serología de hepatitis B y C, determinación de inmunoglobulinas, complemento, creatinina sérica, función renal, urianálisis, estudios de imágenes y biopsia. El tratamiento se basa primariamente en el uso de corticosteroides e inmunosupresores.

Lupus arthropathy: a case series of patients with rhupus.

Among the clinical manifestations of systemic lupus erythematosus (SLE) is an arthropathy, which is usually nonerosive. In many cases the joint involvement is mild. A subset of patients have deforming, nonerosive Jaccoud's arthropathy, and a minority have an arthropathy with clinical findings similar to rheumatoid arthritis (RA) that has been called "rhupus." We report our series of eight patients (seven female, one male) with rhupus arthropathy. Patients were between the ages of 17 and 38 years (average: 30.3 years) at disease onset. All had deforming or Jaccoud's arthropathy, and three had erosive disease. The arthritis was typically the first disease manifestation. Other symptoms of lupus including vasculitis and glomerulonephritis appeared after an average of 2.8 years. All had positive antinuclear antibody and rheumatoid factor. Rhupus arthritis is not a combination of RA and SLE, but should be regarded as a variant of the arthropathy of lupus.

An historical review of systemic lupus erythematosus in Latin America.

In this paper we highlight the role and contributions of Latin American researchers in the study of systemic lupus erythematosus (SLE). The first case of SLE in the world was probably from a mummy from the Huary culture in Peru from about 890 AD, which was extensively studied by macroscopic examination and with light and electronic microscopy, as well as conventional radiography. From the end of the 19th century to the present date there have been many contributions of Latin Americans to the knowledge base of SLE. In this article, we discuss the initial observations of SLE in Latin America and then more specifically review the most important contributions to knowledge about SLE made by physicians in individual Latin American countries, as well as the Gladel group (Latin American group for the study of SLE). The investigation of lupus continues to be pursued by a very dedicated group of Latin American researchers, who continue to enhance our understanding of the pathogenesis and treatment of this condition.

Lupus arthropathy: historical evolution from deforming arthritis to rhupus.

Systemic lupus erythematosus is an autoimmune and inflammatory disease with multiple clinical manifestations, including arthropathy. The clinical presentation of articular involvement is variable, ranging from arthralgia without erosions or deformity to an erosive arthropathy and severe functional disability. A subset of patients with this articular involvement have Jaccoud's arthropathy, and others have an arthropathy with clinical findings similar to rheumatoid arthritis that has been called "rhupus." In this paper we review the historical evolution of concepts of lupus arthropathy, from deforming arthritis to rhupus, and conclude that rhupus is not a combination of rheumatoid arthritis and lupus. Instead, rhupus arthropathy should be regarded as a variant of the arthropathy of systemic lupus erythematosus.

Estudio epidemiológico y de características clinicas de la granulomatosis de Wegener (WG) desde la introducción de la prueba de anticuerpos contra citoplasma de neutrófilos (ANCA) / A population based study of the epidemiology and clinical features of Wegener's granulomatosis since the introduction of anca testing

Desde el advenimiento de la amplia utilización de las pruebas de ANCA, la WG continúa siendo rara. El compromiso de diferentes órganos en esta serie, es similar a lo encontrado en otras series hospitalarias más antiguas. La prueba c-ANCA fue 100 por ciento especifica para el diagnóstico de la WG en esta población.

Declining use of orthopedic surgery in patients with rheumatoid arthritis? Results of a long-term, population-based assessment.

OBJECTIVE: To describe the use of orthopedic surgery, including joint replacement surgery, in a well-defined, population-based cohort of patients with rheumatoid arthritis (RA) and to identify characteristics that predict such use. METHODS: A retrospective medical record review was performed of cases of RA incident in Rochester, Minnesota, during the years 1955-1995. All joint surgeries were recorded. RESULTS: Of the total 609 RA incident cases, 242 patients underwent 1 or more (maximum of 20/patient) surgical procedures involving joints during their followup. Overall, this RA cohort had 7.4 surgeries per 100 person-years of followup; the cumulative incidence for joint surgery for RA-related joint disease at 30 years was 33.7% +/- SEM 3.8%. The risk of having a disease-related joint surgery for RA is increased in patients who are women, younger, positive for rheumatoid factor, and have rheumatoid nodules. When adjusted for duration of followup, patients with RA diagnosed after 1985 were significantly less likely to have undergone joint surgery for RA (P < 0.001). Survival of patients who underwent total joint arthroplasty was similar to those who did not. CONCLUSION: Reconstructive surgeries are common in RA, although patients diagnosed after 1985 are less likely to require joint surgery. These findings may reflect trends in medical disease management and have importance for health care resource utilization planning.

Historia de las vasculitis / History of vasculitis

En este artículo hacemos una completa revisión sobre la historia de las vasculitis, desde los primeros trabajos que mencionaron las manifestaciones clínicas y algunas veces las histológicas de las vasculitis, sin haberse reconocido históricamente la importancia de muchos de estos, hasta los más recientes avances en el conocimiento de estas enfermedades. Es la primera entrega que analiza la historia de las vasculitis desde Hipócrates hasta Buerger